[Extracorporeal photopheresis in progressive systemic sclerosis: discrimination of responders and non-responders].

Abstract

BACKGROUND:

The benefit of extracorporeal photopheresis (ExP) in progressive systemic sclerosis (PSS) is controversial. There is limited experience with the long-term use of ExP in PSS. The purpose of the present study was to distinguish between responders and non-responders by using ExP in PSS and to evaluate activation markers for PSS.

PATIENTS AND METHODS:

20 subjects with PSS were treated for 12 months with ExP (interval: 1x/month) as an immunomodulating monotherapy (11/20) or combination therapy (9/20). The course of PSS was assessed by both specially designed clinical score and serological parameters (CRP, ANA, beta-galactosidase, P-III-P, CD4/CD8-ratio, TNF-alpha, 11-2-R, 11-6).

RESULTS:

After 12 cycles of ExP, 30% of the subjects showed a partial remission and 25%, stable disease (55% responders) while 45% had a progression (non-responders). Although there was no correlation between the clinical course and the serological parameters, an increase of beta-galactosidase during therapy marked a progression of PSS in non-responders. Responders with a short PSS-course before ExP, moderate ANA titres, normal TNF-alpha levels and lack of Scl-70 had a good prognosis.

CONCLUSIONS:

About the half of the subjects with PSS profited by the long-term use of ExP. Thereby the mild immunomodulating effect of ExP seems to be insufficient to control markedly progressive courses of PSS.

• Authors: Stefanie Reich, Michael Radenhausen, Peter Altmeyer, Klaus Hoffmann
• Journal: Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG (J Dtsch Dermatol Ges) Vol. 1 Issue 12 Pg. 945-51 (Dec 2003) ISSN: 1610-0379 [Print] Germany
• Vernacular Title: Die Extrakorporale Photopherese bei Progressiver Systemischer Sklerodermie: Unterscheidung von Respondern und Non-respondern.
• PMID: 16285646 (Publication Type: Comparative Study, Evaluation Study, Journal Article)

Chemical References

• beta-Galactosidase

Topics

• Adult
• Aged
• CREST Syndrome (diagnosis, therapy)
• Data Interpretation, Statistical
• Diagnosis, Differential
• Disease Progression
• Female
• Humans
• Male
• Middle Aged
• Multivariate Analysis
• Photopheresis
• Prognosis
• Prospective Studies
• Remission Induction
• Scleroderma, Systemic (blood, diagnosis, enzymology, therapy)
• Time Factors
• beta-Galactosidase (blood)